{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,21]],"date-time":"2026-09-21T04:13:16Z","timestamp":1789963996806,"version":"4.0.1"},"reference-count":28,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2025,8,20]],"date-time":"2025-08-20T00:00:00Z","timestamp":1755648000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Develop Med Child Neuro"],"published-print":{"date-parts":[[2025,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Dravet syndrome is a severe developmental and epileptic encephalopathy caused by heterozygous, loss\u2010of\u2010function, pathogenic variants in the\n                    <jats:italic>SCN1A<\/jats:italic>\n                    gene. It is characterized by frequent, often prolonged convulsive seizures. Dravet syndrome is associated with diverse comorbid conditions, including developmental impairment, intellectual disability, and behavioral disturbances, in addition to high mortality rates. Prompt diagnosis and treatment initiation are essential to reduce seizure frequency, status epilepticus, and potentially improve neurodevelopmental outcomes. Currently available therapies such as fenfluramine, stiripentol, and cannabidiol have demonstrated significant reduction in convulsive seizure frequency, based on randomized controlled trials and real\u2010world studies; however, they have not markedly affected non\u2010seizure symptoms. Emerging therapies such as genetic modulation are promising given their potential for disease modification, seizure frequency reduction, and improvement of non\u2010seizure outcomes. This review of observational studies and randomized clinical trials provides an overview of the natural history of Dravet syndrome, current treatment guidelines with recently approved medications, addressing both safety and efficacy, and new therapeutic approaches, including disease\u2010modifying therapies. The therapeutic landscape in Dravet syndrome is evolving, with the use of new antiseizure agents and precision therapies that target the\n                    <jats:italic>SCN1A<\/jats:italic>\n                    variant. Goals of treatment include improvement of seizure burden, neurodevelopment, comorbidities, and quality of life in individuals with Dravet syndrome.\n                  <\/jats:p>","DOI":"10.1111\/dmcn.16475","type":"journal-article","created":{"date-parts":[[2025,8,21]],"date-time":"2025-08-21T05:54:48Z","timestamp":1755755688000},"page":"1527-1535","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["State\u2010of\u2010the\u2010art management of Dravet syndrome"],"prefix":"10.1111","volume":"67","author":[{"given":"Alejandra","family":"Vasquez","sequence":"first","affiliation":[{"name":"Department of Neurology Mayo Clinic  Rochester MN USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3015-8282","authenticated-orcid":false,"given":"Elaine C.","family":"Wirrell","sequence":"additional","affiliation":[{"name":"Department of Neurology Mayo Clinic  Rochester MN USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,8,20]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-8749.2011.03964.x"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.17015"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/aws151"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awac210"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000200028"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.yebeh.2023.109604"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.17850"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.yebeh.2022.108661"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.yebeh.2024.110102"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/dmcn.13727"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.17274"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(19)32500-0"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamaneurol.2019.4113"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.17854"},{"key":"e_1_2_9_16_1","article-title":"Fenfluramine treatment for Dravet syndrome: Long term real\u2010world analysis demonstrates safety and reduced health care burden","author":"Boncristiano A","year":"2024","journal-title":"Epilepsia"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(00)03157-3"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1007\/s40120-024-00623-8"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1111\/dmcn.15638"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1611618"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamaneurol.2020.0073"},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.18308"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1167.2005.05705.x"},{"key":"e_1_2_9_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pediatrneurol.2024.12.012"},{"key":"e_1_2_9_25_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.17737"},{"key":"e_1_2_9_26_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199708283370901"},{"key":"e_1_2_9_27_1","article-title":"Emerging Genetic Therapies in Epilepsy","author":"Sullivan J","year":"2025","journal-title":"Semin Neurol"},{"key":"e_1_2_9_28_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2018.10.023"},{"key":"e_1_2_9_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.yebeh.2018.01.003"}],"container-title":["Developmental Medicine &amp; 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